Ventricular Lesions

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Lateral ventricular lesions

By

Dr.Islam Alaghory
Lecturer of neurosurgery
Alazhar University
Intraventricular neoplasms are about 10% of all
intracranial neoplasm

and arise from periventricular structures such as

,ventricular system the walls of the

the septum pellucidum and

 .choroid plexus the


Many tumors arise from, or can bulge into the 

ventricular system, although there are certain


lesions that are relatively restricted to
 .ventricles

A number of factors assist in defining the 


differential diagnosis, both radiological and
clinical, including where the lesion is positioned
within the ventricle as well as age and any
associated conditions
Neoplasms of the ventri­cular wall and septum
pellucidum

ependymoma
subependymoma
central neurocytoma
subependymal giant cell astrocytoma
Neoplasms of the choroid plexus 

choroid plexus papilloma 

choroid plexus carcinoma 


Others
intraventricular meningioma
intraventricular metastasis

oligodendroglioma
pilocytic astrocytoma
 glioblastoma
intraventricular CNS lymphoma
medulloblastoma
primitive neuroectodermal tumour
sarcoma
intraventricular teratoma
 Non-neoplastic lesions
colloid cysts
neurocysticercosis
intracranial hydatid cyst
tuberculoma intracranial
AVM
Ependymoma 

s
:Origin 

.spinal cord lining the ventricles of the brain or the central canal of the
:Incidence
of all paediatric brain tumours and up to 33% of brain tumours 10%~ 
 .occurring in those less than 3 years of age
:Site 

Ependymomas can occur anywhere, but certain locations are more


: typical. Overall distribution is
posterior fossa: 60% 

30% :supratentorial ependymoma 

spinal cord/canal: 10% 

spinal cord ependymoma ◦


myxopapillary ependymoma ◦
Ependymomas can occur in any age group but 
are more common in younger patients 
.
Those that occur in the posterior fossa are 
more common in children (mean age, 6 years),
whereas the mean age for supratentorial
lesions is 18–24 years 

Ependymomas are the third most common brain 

neoplasm in children, behind medulloblastoma


(primitive neuroectodermal tumor) and
astrocytic tumor
Pathology
Ependymomas are glial tumours with ependymal 
differentiation which tend to arise within or
abutting the ventricular system of the brain or
. central canal of the spinal cord

Although for many years ependymomas were 


believed to be tumours arising from
dedifferentiated ependymocytes, it now
appears fairly certain that in fact, they arise
 .  glial stem cells from
Macroscopic appearance 
Macroscopically, ependymomas tend to be well 

.defined lobulated gray and soft mass


 .They may have focal areas of calcification 
Microscopic appearance 
Microscopically, these tumours are characterised 

by well-differentiated cells. Characteristic


features include ependymal rosettes, which are
uncommon but pathognomonic and perivascular
pseudorosettes which are far more common and
. seen in most of ependymomas
Dystrophic calcification, haemorrhage, myxoid 
degeneration and even rarely metaplasia (bone or
 . cartilage) are sometimes encountered
Ependymal rosettes (ependymoma)
Immunohistochemistry 
glial fibrillary acid protein (GFAP) 

around the perivascular pseudorosettes

epithelial membrane antigen (EMA) 

positive in the luminal surface of the ependymal rosettes ◦

positive :S100 

positive :vimentin 
Classification
WHO grade I 
  subependymoma ◦
  myxopapillary ependymoma ◦

WHO grade II 
papillary ependymoma 
clear cell ependymoma 
 
WHO grade III 
anaplastic ependymoma ◦
Radiographic features
Ependymomas are typically 

heterogeneous masses with areas of


necrosis, calcification, cystic change and
haemorrhage frequently seen. This results in a
 .heterogeneous appearance on all modalities
CT
Brain 

 coarse calcification is common (50%) 

 cystic areas (50%) 


solid component iso to hypodense 
 heterogeneous enhancement 
variable haemorrhage 
 .
Intraventricular lesions may extend into adjacent
brain, and there may be vasogenic edema in the
adjacent periventricular white matter
MRI 
Brain 

T1 

solid portions of ependymoma ◦


typically are isointense to hypointense ◦
relative to white matter ◦

T2 

hyperintense to ◦
white matter ◦

T1 C+ (Gd) 

heterogeneous ◦
DWI/ADC
restricted diffusion may
be seen in solid
components, especially in anaplastic
tumour

MRS
Choline peak elevation according
to the cellularity of tumour
Differential diagnosis

 medulloblastoma 

similar demographic, especially ◦


if around the 4th ventricle ◦
arises from vermis ◦
less 'plastic', does not tend ◦
to extend through foramina ◦
enhancement more homogenous ◦
 calcification less common ◦
subependymoma 

tends to occur in older individuals ◦


choroid plexus papilloma 

in children usually in the trigone of the lateral ventricles ◦


in adults usually in the fourth ventricle (i.e opposite to ◦
ependymoma)
more vividly and homogeneously enhancing ◦
lacks adjacent parenchymal oedema ◦
central neurocytoma 

usually arises from/in contact with ◦


septum pellucidum less vivid enhancement ◦
Subependymomas
Subependymomas account for 0.2%–0.7% of 
intracranial neoplasms; however, this may be an
underestimate, since often they are asympto-
matic and found incidentally

Thought that they arise from the  


subependymal glial layer surrounding the
cerebral ventricles, but the exact histogenesis
remains uncertain
Subependymomas

Subependymomas are uncommon, benign (WHO grade I) 

 tumours which are slow growing and non-invasive. They


tend to occur in middle-aged and older individuals and
 .usually identified as an incidental finding

Terminology 

These tumours were previously also known as subependymal


astrocytomas, not to be confused with subependymal
giant cell astrocytomas, which are both seen in association
with tuberous sclerosis. They are also considered by some to
be variants of ependymomas, with which they may co-exist
Epidemiology 
Subependymomas tend to present in middle- 
aged to older individuals (typically 5th to 6th
decades 3). There is a slight male predilection
(M:F 2.3:1)
Clinical presentation 
Typically patients are asymptomatic and small 
lesions are discovered incidentally. In some
cases, especially when the tumours are larger,
presentation is with symptoms of raised
intracranial pressure due to
obstructive hydrocephalus
Pathology

Macroscopic appearance 
.nodules, < 2cm , narrow pedicle
Size is the most important distinguishing feature
.compared to subependymal giant cell astrocytoma
Microscopic appearance 
subependymal glial layer with low cellularity 
. and no high-grade features
Occasionally foci of cellular ependymoma are 
. seen
Radiographic features

fourth ventricle: 50-60% 

:lateral ventricles (usually frontal horns) 

30-40% 

third ventricle: rare 


central canal of the spinal cord: rare
CT 

Isodense . If large, it may have cystic or even calcific (up to half of 

.the cases 3) components.no vasogenic oedema


MRI 

T1 

iso-hypointense ◦
generally homogeneous but may be heterogeneous ◦
in larger lesions ◦
T2 

,hyperintense , heterogeneity may be seen in larger lesions ◦



T1 C+ (Gd) 

usually no enhancement, although at ◦


times may demonstrate ◦
mild enhancement ◦
DSA-angiography 

.show no or little vascularity 6 


Treatment and prognosis

If appearances are characteristic and the 


patient is asymptomatic, then follow up is a
.viable option
Resection should be considered if the patient is 

symptomatic (hydrocephalus or mass effect),


the mass has an atypical appearance or
demonstrates growth. Local resection is
. curative
Central 

  neurocytomas
Central neurocytomas account for 0.25%–0.5% 

of intracranial tumors

The origin of these tumors is unclear, but 


they arise from bipotential progenitor cells 

that are capable of both neuronal and glial


differentiation
 Epidemiology (70% diagnosed between 20 and 40
years of age) and
 no reported gender predilection 10. 

 Clinical presentation
 Typically, central neurocytomas present with
symptoms of increased intracranial pressure,
headaches being most frequent, or seizures
(especially tumours with extraventricular extension).
 A relatively short clinical course, typically only a
few months, is most common. . 
.WHO grade II lesions 

The initial description classified them as WHO 

grade I lesions. However, this was upgraded in


1993 to WHO grade II as it was recognised
that at least some of these tumours exhibited
more aggressive behaviour
Imaging Features
well-circumscribed, lobulated masses that 
frequently have cystlike areas. Up to 50% may
contain calcification, and hemorrhage may
.rarely be seen

At CT, these lesions are hyperattenuating 


Central neurocytoma .  Axial nonenhanced CT image shows a lesion
located centrally within the lateral ventricles. Small foci of
.calcification , as cystic areas
:MRI 
isointense to gray matter on T1-weighted images 

. and hyperintense on T2-weighted images

These lesions may have a “bubbly” appearance 

due to the presence of multiple cysts 


 T1 C+

◦ mild-moderate heterogeneous enhancement


.
Axial contrast-enhanced T1-
 .Axial T2-weighted image weighted image shows moderate
  heterogeneous enhancement
 MR spectroscopy
◦ may have a strong choline peak
glycine peak (3.55ppm) has also been 

reported

 Treatment and prognosis


Complete surgical resection is usually curative 
.
Subependymal

Giant Cell Tumor


Subependymal Giant Cell Tumor
most common cerebral neoplasm 
. in patients with tuberous sclerosis (TS) 

It has previously been referred to as subependymal 

giant cell astrocytoma, but recent pathologic


studies have revealed that it is a glioneuronal
tumor; thus, the trend is away from use of the
term subependymal giant cell astrocytoma
SGCT is considered pathognomonic for TS, but 

there have been rare reports of SGCT in


. patients without manifestations of TS
There is a wide age range for presentation, from 

. birth to the 5th decade (mean age, 11 years)

These lesions arise near the foramen of Monro (F, 

and it has been proposed that SGCTs arise from a


. subependymal nodule

They are slow-growing lesions, and due to their 


location they commonly manifest with symptoms of
increased intracranial pressure from obstructive
.hydrocephalus
Autopsy photograph of a brain shows a mass
located adjacent to the foramen of Monro
Pathologic Findings
SGCT is a WHO grade I 
well-circumscribed solid intraventricular 

.near the foramen of Monro 

The histogenesis of SGCT is unclear, but there 

is evidence to support both neuronal and


.astrocytic feature

Immunohistochemical staining reveals markers 

for both glial and neuronal proteins


Imaging Features
Imaging reveals a well-circumscribed lesion 
that is typically larger than 1 cm and most
commonly located near the foramen of Monro.
.Other subependymal nodules may be noted

 
.At CT, SGCTs are hypo- to isoattenuating

MR imaging reveals a lesion that is hypo- to


isointense to gray matter on T1-weighted
images and iso- to hyperintense on T2-
.weighted images

At contrast-enhanced imaging, the lesions


avidly enhance
 .
Axial T2-weighted image shows a hyperintense mass near the foramen of Monro.  )a(
Multiple subcortical focal areas of T2 hyperintensity are noted, which are consistent with
subcortical tubers.
 (b) Coronal contrast-enhanced T1-weighted image shows avid enhancement of the

.neoplasm
Indications for resection include increasing 
tumor size, hydrocephalus, a new focal
neurologic deficit, or symptoms of increased
.intracranial pressure

Once an SGCT is identified, follow-up imaging 

. at yearly intervals is recommended


Choroid plexus tumours
choroid plexus papilloma (CPP) ◦
80% 

and WHO Grade II when atypical ,WHO Grade I 

choroid plexus carcinoma (CPC)  ◦


20% 
WHO Grade III 
Radiographic features 

On imaging choroid plexus papillomas are 


characterised by vividly enhancing, usually
intraventricular, masses. Hydrocephalus is common

The distribution of tumours matches the distribution


:of choroid
50% :lateral ventricle 

40% :fourth ventricle 

5% :third ventricle 

multicentric: 5 
Choroid Plexus Neoplasms
Choroid plexus tumors account for 2%–4% of
pediatric brain tumors, 0.5% of adult brain
tumors, and up to 20% of pediatric neoplasms
. occurring in the 1st year of life

These neoplasms arise anywhere that choroid


plexus is located and develop from the choroid
plexus epithelium
presentation
,Patients frequently present with hydrocephalus 

however, CPCs may also manifest with focal


.neurologic deficits
Pathologic Findings
CPP is histologically similar to normal choroid
plexus, consisting of bland cuboidal to columnar
epithelial cells surrounding a delicate
.fibrovascular stalk

Atypical CPPs demonstrate two or more mitoses


.per 10 randomly selected high-power fields
 
Diagnosis of CPC requires the presence
of at least four of the following
features: more than five mitoses per
high-power field, increased cellular
density, nuclear pleomorphism and
necrosis
Imaging Features
Choroid plexus neoplasms are very
vascular lesions that demonstrate avid
enhancement at contrast-enhanced
.imaging
They are iso- to hyperattenuating at CT.
CT angiography as well as conventional
angiography may reveal an enlarged
choroidal artery if the neoplasm is in the
atrium of the lateral ventricle
CPP . (a) Axial nonenhanced CT image shows a lobulated slightly
hyperattenuating mass in the atrium of the right lateral ventricle.
Hydrocephalus is present. (b) Axial contrast-enhanced T1-weighted
image shows avid enhancement of the mass
 Axial T2-weighted image shows a large heterogeneous mass with cystic and solid components arising from
the lateral ventricle with surrounding vasogenic edema.  
 Sagittal contrast-enhanced T1-weighted image shows an enhancing nodulealong the cauda equina, a finding
consistent with CSF dissemination.
MR spectroscopy of choroid plexus tumors 
demonstrates a marked choline peak without
;an N-acetylasparate or creatine peak

carcinomas also demonstrate elevation of the 

lactate leve
treatment and prognosis 
. Total excision should be the aim 
Overall there is 90% 1-year-survival, and 77% 

 . 5-year-survival

CSF seeding is uncommon in choroid plexus 


papillomas, but far more frequently seen in
 . choroid plexus carcinomas
Intraventricular meningiomas
Epidemiology
.represent between 0.5 and 2% of all meningiomas4
between the 4th and 6th decades 
.M:F ratio of 1:2 4
Clinical presentation 
Intraventricular meningiomas present usually due 
to mass effect, either by direct compression of
the adjacent brain, or from obstruction to normal
hydrocephalus CSF drainage with resultant
Pathology 
lateral ventricle trigone of 80% 

arise from mengingothelial inclusion bodies 

located in the tela choroidea and/or


.  choroid plexus mesenchymal stroma of the

In general, these meningiomas are most 

 fibrous meningiomas commonly of the


Syndrome of the trigone
occurs when intracranial tumours that arise in the 
trigone of the lateral ventricle present with a
distinctive constellation of signs and symptoms due
to compression of the posterior limb of the
internal capsule and the adjacent white matter of
the parietal and posterior frontal cortex. Clinically
: this manifests as

contralateral limb weakness 

contralateral homonymous hemianopsia 

 receptive dysphasia 
If the mass grows downward far enough to
compress the superior cerebellar peduncle then
.ipsilateral cerebellar signs may also be present

Non specific symptoms may of course also co-


exist, such as headaches, seizures and
.cognitive changes
Radiographic features

isodensity and intensity to grey matter


precontrast and vivid, usually homogenous
.enhancement following administration of contrast
Axial nonenhanced CT image shows a hyperattenuating mass in the atrium of the )a(  
right lateral ventricle. (b) Axial T2-weighted image shows a slightly heterogeneous,
.predominantly isointense mass. No surrounding edema is present
Axial diffusion-weighted image shows foci of reduced
diffusion.  Axial contrast-enhanced T1-weighted image shows
.avid homogeneous enhancement
MR spectroscopy reveals elevated choline
.level with variable amounts of lactate
Metastases
Metastases
Intraventricular metastases account for 0.9%–
. 4.6% of cerebral metastases

In adults, renal, colon, and lung carcinoma are


the most common causes; in children,
neuroblastoma, Wilms tumor, and retinoblastoma
. are most common

 
Intraventricular metastases are most common
in the lateral ventricles but may also occur in
the third ventricle and very rarely in the
.fourth ventricle
 
Avid enhancement is usually seen on contrast-
enhanced images, and vasogenic edema may be
seen in the adjacent brain parenchyma
Metastasis: shows diffuse ependymal enhancement and
hyperdense nodule in the left paraventricular region with edema in patient
of carcinoma thyroid
PENT
The term has fallen out of favour and has been 

removed from the 2016 update to the


WHO classification of CNS tumours, now
incorporated into the term embryonal tumours
 . with multilayered rosettes (ETMR) 10
Traditionally a number of tumours variably fell 

: under the umbrella term CNS PNET


by far the most common :medulloblastoma 
supratentorial PNET: 15% of all CNS PNET 
  most common supratentorial PNET :pineoblastoma ◦
:ependymoblastoma ◦
  medulloepithelioma ◦
rare :spinal PNET ◦
Thank
you

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